Article
Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice.
Human molecular genetics - 1 Mar 2000
Wheeler V C, White J K, Gutekunst C A, Vrbanac V, Weaver M, Li X J, Li S H, Yi H, Vonsattel J P, Gusella J F, Hersch S, Auerbach W, Joyner A L, MacDonald M E
Abstract excerpt
Huntington's disease (HD) is caused by an expanded N-terminal glutamine tract that endows huntingtin with a striatal-selective structural property ultimately toxic to medium spiny neurons. In precise genetic models of juvenile HD, HdhQ92 and HdhQ111 knock-in mice, long polyglutamine segments change huntingtin's physical properties, producing HD-like in vivo correlates in the striatum, including nuclear...
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